Causes of Spontaneous Scleral Hemorrhage
Spontaneous scleral hemorrhage most commonly results from vascular abnormalities, coagulation disorders, or systemic conditions such as hypertension, with the specific etiology determining management approach and prognosis.
Primary Causes
Vascular Abnormalities
- Arteriovenous malformations (AVMs)
- Arteriovenous fistulas
- Cavernous malformations
- Aneurysms (particularly in adolescents and adults)
- Moyamoya syndrome with fragile collateral vessels
Hematologic and Coagulation Disorders
- Thrombocytopenia (particularly when platelet count falls below 20,000/mm³)
- Factor deficiencies (particularly Factor VIII, XIII)
- Vitamin K deficiency
- Anticoagulant therapy (warfarin, direct oral anticoagulants)
- Antiplatelet therapy
- Protein C and Protein S deficiencies
- Liver disease with associated coagulopathy
Systemic Conditions
- Uncontrolled hypertension 1, 2
- Diabetes mellitus with associated vascular changes 3
- Sickle cell disease (SCD) 4
- SCD can cause various types of hemorrhages including subconjunctival and scleral bleeding
- Often associated with vascular fragility in younger patients
- In adolescents and adults, may be related to aneurysms
Other Causes
- Malignant tumors (hemorrhage into tumor region) 4
- Cocaine and sympathomimetic drug use 4
- Recent transfusion (particularly in SCD patients) 4
- Corticosteroid use (particularly in SCD patients) 4
Risk Factors for Spontaneous Scleral Hemorrhage
Patient-Related Factors
- Advanced age
- History of prior hemorrhages
- Family history of hemorrhagic disorders
- Apolipoprotein E genotypes (ε2 or ε4 alleles) 4
Medication-Related Factors
- Intensity of anticoagulation (higher INR values increase risk)
- Combination anticoagulant and antiplatelet therapy 5
- Duration of anticoagulant therapy
Diagnostic Approach
Initial Assessment
- Detailed medication history (anticoagulants, antiplatelets, decongestants, sympathomimetics) 4
- Blood pressure measurement (hypertension is a common cause)
- Complete blood count with platelet count
- Coagulation studies (PT/INR, aPTT)
- Assessment for signs of liver disease
Imaging Considerations
- Ocular ultrasound to evaluate extent of hemorrhage and identify underlying structural abnormalities
- Consider MRI if vascular malformation is suspected
- CT angiography or MR angiography if arteriovenous malformation or aneurysm is suspected
Management Implications
The underlying cause of spontaneous scleral hemorrhage determines management:
- Coagulation disorders: May require factor replacement or reversal of anticoagulation
- Hypertension: Requires blood pressure control
- Vascular malformations: May require surgical intervention
- Drug-induced: Cessation of causative agent
Clinical Pearls and Pitfalls
- Pearl: In patients with SCD and scleral hemorrhage, consider underlying aneurysm, especially in adolescents and adults 4
- Pitfall: Assuming all scleral hemorrhages are benign - thorough evaluation is needed to rule out serious underlying causes
- Pearl: The severity of coagulation abnormalities correlates with risk - more severe deficiencies carry higher risk 4
- Pitfall: Failing to check for sympathomimetic drug use in young or middle-aged patients with unexplained hemorrhage 4
In patients with spontaneous scleral hemorrhage, a systematic approach to identify the underlying cause is essential for appropriate management and prevention of recurrence. The presence of certain risk factors should prompt more thorough investigation for specific etiologies.